Orthotopic liver transplantation is being performed for patients with non-neoplastic end stage liver disease. Included are liver diseases associated with inborn errors of metabolism such as Wilson's disease, congenital tyrosinemia, alpha1-antitrypsin deficiency and alpha-glucosidase deficiency. Postoperatively, patients with inborn errors of metabolism are being studied with specific biochemical markers to ascertain if metabolic cure has been achieved. Correction of the metabolic errors has been documented in all inborn errors cited above. General improvements in care of all patients are being instituted, including new techniques of biliary tract reconstruction. The immunosuppressive regimen to prevent rejection has been modified to include long-term thoracic duct fistula which is instituted at the time of transplantation. Further work is being carried out to define the specific hepatotrophic effects of portal venous blood and to apply all lessons learned in transplantation to traditional hepatic surgery such as subtotal hepatectomy portacaval shunt.